Hemophilia. Causes, symptoms, types, diagnosis and treatment of hemophilia.

The two main tasks of hemostasis are to maintain blood in a fluid state and to prevent its loss in the event of injury. The operation of the system looks like a chain of complex biochemical reactions in which blood cells, plasma and vessel walls participate.

Bleeding disorders can be inherited. In some cases, they are acquired through medications, malfunctions of the immune system, malignant processes and liver diseases, in which the production and exchange of coagulation factors occurs.

All problems associated with bleeding disorders

Problems associated with bleeding disorders are usually caused by abnormal blood flow to block blood vessels when they are injured.
There are also genetic abnormalities that complicate the process of blood clotting. If everything is normal in the body, the blood at the site of the cut should thicken, that is, prevent heavy bleeding. However, in some cases, this mechanism does not work, which leads to large blood losses.

In addition, with poor blood clotting, its external loss does not always occur. Such disorders can manifest themselves in the form of internal bleeding, for example under the skin, causing hematomas, and even into the brain.

Causes of bleeding disorders

Typically, poor blood clotting is caused by a deficiency of anticoagulants and other substances that ensure normal blood clotting. Such substances include proteins. In the presence of certain diseases, there may be no proteins in the plasma at all. As a rule, such diseases are hereditary, for example, hemophilia.

However, abnormal blood clotting is caused not only by genetic abnormalities, but also by several other factors, such as:

• Lack of vitamin K in the body.

• Hereditary diseases: von Willebrand disease and hemophilia.

• Liver carcinoma or the presence of metastases from other organs affected by cancer.

• Infectious liver diseases, such as hepatitis.

• Diseases that cause scarring on the liver (cirrhosis).

• Long-term use of strong antibiotics or drugs aimed at combating blood clots.

• The use of anthogenesis inhibitors - drugs that slow down the development of new blood vessels.

• Thrombocytopenia is a disease in which the concentration of platelets drops to a minimum level.

• Anemia of various types.

Under control

Article on the topic

Useful tips from Tatyana Ressina: Do not stop bleeding with hydrogen peroxide! Fortunately, recently our hematologists have had such an opportunity. Since 2005, the state has taken care of the treatment of a number of such patients.

Previously, the presence of a severe disease of the blood coagulation system was a death sentence for its owner, and the life expectancy of patients with hemophilia, for example, was only 30–34 years. This disease was treated with plasma transfusions, after which those suffering from hemophilia, in addition to one serious illness, often received another - hepatitis C. Others became disabled due to hemorrhages that deformed and destroyed joints. But thanks to the state federal program “7 nosologies”, which included the treatment of a number of the most expensive diseases (including blood), there are much fewer such neglected patients in our country. And their hospitalization decreased by 4 times.

By the way

Blood clotting is a protective reaction of the body that prevents blood loss during injuries and other life-threatening conditions. This process is a sequential biochemical reaction that occurs with the participation of blood clotting factors (a number of plasma proteins and calcium ions). To date, 13 blood clotting factors are known, each of which is designated by Roman numerals. And how many children were saved! After all, if you start taking the necessary medications on time, a child with hemophilia can grow up absolutely healthy: go to school, play sports. And when in 2007 a group of teenagers with severe hemophilia climbed Elbrus, for thousands of patients and their parents it was not just a victory. For them, this meant a change from an era of despair to an era of hope.

Of course, such patients have to give themselves intravenous injections of the missing blood clotting factor for life, avoid injuries, blood-thinning drugs containing aspirin, NSAIDs, and carefully undergo vaccinations and other invasive medical procedures and procedures. But otherwise, the lives of people with hereditary and acquired diseases of the blood coagulation system today are practically no different from the lives of their healthy peers. The main thing is to identify the disease in time and keep it under control.

Treatment of bleeding disorders

Treatment of bleeding disorders is carried out according to the reasons that cause such an anomaly. If the disorder is caused by the presence of liver cancer, then first all therapeutic measures should be taken to remove the tumors, and then resort to medication to restore normal blood clotting. In addition, there are general methods of treating this disorder:

• Vitamin K injection.

• Transfusion of donor platelets or plasma.

• Taking medications aimed at restoring blood clotting function.

• Taking iron supplements.

• Treatment with Oprelvekin and hydroxyurea.

References

  1. Solyanik, O.V., Ivanko, O.G. Modern ideas about the role of vitamin K in the physiology and pathology of children, 2014. - No. 3 (47).
  2. Kodentsova, V.M. Vitamin K: functional role and food sources, 2021. - No. 4.
  3. Alexandrova, E.V., Skoda, A.S., Yurchenko, D.N. and others. Biochemical foundations of vitaminology, 2015. - 129 p.
  4. Gordeeva, O.B., Tepaev, R.F., Botvinyeva, V.V. and others. Vitamin K-dependent hemorrhagic conditions in children after the neonatal period. Hematology and transfusiology, 2021. - No. 1.

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